Name: U-Blot® ACADV Rabbit mAb
SKU: WR6042
Price: 268 USD
Availability: InStock

U-Blot® ACADV Rabbit mAb

Ucallm_WR6042-50

Price range: $268.00 through $328.00

Size

100μl

50μl

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SKU: WR6042-50

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Product details

Background：
acyl-CoA dehydrogenase , very long chain (ACADVL) Homo sapiens The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq , Jul 2008] ,

Specifications

Size	50μL/100μL
Target	ACADV
Reactivity	Human
Applications	WB, IHC, IF, IP, ELISA
MW(Calculated)	70kD
MW(Observed)	67kD
Host Species	Rabbit
Isotype	IgG,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Purification	Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	RM2014R
Immunogen	The specific immunogen used to produce this antibody is proprietary information.
Specificity	Endogenous
Gene Name	ACADVL VLCAD
Protein Name	Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) (EC 1.3.8.9)
Other Name
Species	Human
Gene ID	37
Uniprot	P49748,
Species.1	Mouse
Gene ID.1
Uniprot.1	P50544,
Species.2	Rat
Gene ID.2
Uniprot.2	P45953
Cellular Localization	Mitochondrion inner membrane; Peripheral membrane protein .; [Isoform 2]: Mitochondrion inner membrane; Peripheral membrane protein .
Tissue Expression	Adipose tissue,Liver,Lung,Pancreas,Peripheral blood,Placenta,
Signaling_pathway
Research Areas	>>Fatty acid degradation; / >>Metabolic pathways; / >>Fatty acid metabolism; / >>Alcoholic liver disease
Function	Catalytic activity:Acyl-CoA + ETF = 2,3-dehydroacyl-CoA + reduced ETF.,cofactor:FAD.,Disease:Defects in ACADVL are the cause of very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency) [MIM:201475]. VLCAD deficiency is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.,Function:Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.,miscellaneous:A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.,pathway:Lipid metabolism; mitochondrial fatty acid beta-oxidation.,similarity:Belongs to the acyl-CoA dehydrogenase family.,subunit:Homodimer.,